Suh Mi Ri, Choi Won Ah, Kim Dong Hyun, Lee Jang Woo, Kim Eun Young, Kang Seong-Woong
Department of Rehabilitation Medicine, Gangnam Severance Hospital, Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, South Korea.
Department of Rehabilitation Medicine, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, South Korea.
Respir Care. 2018 Mar;63(3):274-281. doi: 10.4187/respcare.05475. Epub 2018 Jan 3.
The purpose of this study was to investigate the 5-year outcomes of noninvasive ventilation (NIV) application in different neuromuscular disease (NMD) groups.
We categorized 180 subjects who had initiated NIV between March 2001 and August 2009 into 4 groups and followed them for > 5 y. The NIV maintenance rate and average duration, applying time, and forced vital capacity (FVC) were investigated at the time NIV was initiated and 5 y after NIV initiation in each group.
In subjects with amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and spinal muscular atrophy (SMA)-congenital myopathy, the 5-year subjects who continued to use NIV over time were 22.5%, 89.4%, and 91.3%, respectively, and the average NIV maintenance durations were 21.53 ± 19.26 months, 55.22 ± 11.47 months, and 57.48 ± 8.34 months, respectively ( < .001). Median daily applying time changed from 8.0 h to 24.0 h ( < .001), from 8.0 h to 12.0 h ( < .001), and from 8.0 h to 9.0 h ( = .11) in subjects with ALS, DMD, and SMA-congenital myopathy, respectively. FVC decreased significantly after 5 y except in the group with combined SMA-congenital myopathy.
NIV was tolerated long-term without significant increases in daily application time for most subjects with NMD. However, in individuals with ALS, development of severe bulbar symptoms can risk maintaining NIV.
本研究旨在调查不同神经肌肉疾病（NMD）组无创通气（NIV）应用的5年结局。
我们将2001年3月至2009年8月开始使用NIV的180名受试者分为4组，并对他们进行了超过5年的随访。在每组开始使用NIV时以及开始使用NIV 5年后，调查NIV维持率、平均持续时间、应用时间和用力肺活量（FVC）。
在肌萎缩侧索硬化症（ALS）、杜氏肌营养不良症（DMD）和脊髓性肌萎缩症（SMA）-先天性肌病患者中，随时间推移继续使用NIV的5年患者分别为22.5%、89.4%和91.3%，平均NIV维持持续时间分别为21.53±19.26个月、55.22±11.47个月和57.48±8.34个月（P<0.001）。ALS、DMD和SMA-先天性肌病患者的每日中位应用时间分别从8.0小时变为24.0小时（P<0.001）、从8.0小时变为12.0小时（P<0.001）和从8.0小时变为9.0小时（P = 0.11）。除合并SMA-先天性肌病组外，5年后FVC显著下降。
对于大多数NMD患者，NIV可长期耐受，且每日应用时间无显著增加。然而，在ALS患者中，严重延髓症状的出现可能会影响NIV的维持。
